Abstract:Background:
Cystic fibrosis-related liver disease (CFLD) is the leading nonpulmonary cause of mortality in cystic fibrosis (CF). We evaluated and compared the burden of disease and nonrespiratory comorbidities of those with severe CFLD and those without (noCFLD).
Methods:
A retrospective nationwide (Australia) longitudinal review (from 1998 to 2016) of severe CFLD patients compared with noCFLD controls (matched 1 : 1 for age, genotype, pancreatic insuff… Show more
“…CFLD group had an increased prevalence of other CF comorbidities including respiratory failure, intestinal obstruction, rectal prolapse and constipation, diabetes, and lung transplantation. An Australian study by Singh et al 26 reported that patients with CFLD had increased disease burden requiring multiple hospitalizations for both respiratory and nonrespiratory indications, and were at increased risk for bone disease and diabetes. Thornton et al 8 showed that more than one‐third of patients with cirrhosis had ≥3 comorbidities.…”
Background: Cystic fibrosis-related liver disease (CFLD) is more prevalent in recent decades due to the increasing life expectancy of patients with cystic fibrosis (CF).There is paucity of population-level data on the impact of CFLD on hospital outcomes.
Methods:We interrogated nonoverlapping years (2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014)(2015)(2016) of the National Inpatient Sample and Kids' Inpatient Database to include all hospitalized patients <21 years of age with a primary diagnosis of CF within the United States. A concomitant diagnosis of cirrhosis, liver fibrosis, chronic liver disease, portal hypertension, hepatomegaly, splenomegaly, hypersplenism, and liver transplant status was considered as surrogates for the diagnosis of CFLD and was compared with CF-related hospitalizations without these diagnoses (controls) for demographics, comorbid conditions, in-hospital mortality, length-of-stay, and hospital charges.Results: We evaluated 94,374 CF-related hospitalizations. The prevalence of CFLD was 5.8%. The prevalence increased from 3.1% (2003) to a peak of 7.3% (2014) with an overall increasing trend, p < 0.001. Hospitalizations with CFLD had an increased prevalence of significant comorbidities: respiratory failure,lung transplant, pulmonary hypertension, diabetes mellitus, malnutrition, Clostridioides difficile infection, cholelithiasis, anemia, and need for parenteral nutrition, p < 0.001. Multivariate regression models showed CFLD as independently associated with 2.1 (95% confidence interval [CI]: 1.5 to 2.8) times increased risk of inpatient mortality, contributed to 1.1 (95% CI: 0.89 to 1.37) additional days of hospitalization, and incurring $14,852 (95% CI: 12,204 to 17,501) excess hospital charges, p < 0.001.
Conclusion:CFLD is associated with multiple comorbidities and is independently associated with increased risk of mortality and increased health care resource utilization in pediatric CF-related hospitalizations.
“…CFLD group had an increased prevalence of other CF comorbidities including respiratory failure, intestinal obstruction, rectal prolapse and constipation, diabetes, and lung transplantation. An Australian study by Singh et al 26 reported that patients with CFLD had increased disease burden requiring multiple hospitalizations for both respiratory and nonrespiratory indications, and were at increased risk for bone disease and diabetes. Thornton et al 8 showed that more than one‐third of patients with cirrhosis had ≥3 comorbidities.…”
Background: Cystic fibrosis-related liver disease (CFLD) is more prevalent in recent decades due to the increasing life expectancy of patients with cystic fibrosis (CF).There is paucity of population-level data on the impact of CFLD on hospital outcomes.
Methods:We interrogated nonoverlapping years (2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014)(2015)(2016) of the National Inpatient Sample and Kids' Inpatient Database to include all hospitalized patients <21 years of age with a primary diagnosis of CF within the United States. A concomitant diagnosis of cirrhosis, liver fibrosis, chronic liver disease, portal hypertension, hepatomegaly, splenomegaly, hypersplenism, and liver transplant status was considered as surrogates for the diagnosis of CFLD and was compared with CF-related hospitalizations without these diagnoses (controls) for demographics, comorbid conditions, in-hospital mortality, length-of-stay, and hospital charges.Results: We evaluated 94,374 CF-related hospitalizations. The prevalence of CFLD was 5.8%. The prevalence increased from 3.1% (2003) to a peak of 7.3% (2014) with an overall increasing trend, p < 0.001. Hospitalizations with CFLD had an increased prevalence of significant comorbidities: respiratory failure,lung transplant, pulmonary hypertension, diabetes mellitus, malnutrition, Clostridioides difficile infection, cholelithiasis, anemia, and need for parenteral nutrition, p < 0.001. Multivariate regression models showed CFLD as independently associated with 2.1 (95% confidence interval [CI]: 1.5 to 2.8) times increased risk of inpatient mortality, contributed to 1.1 (95% CI: 0.89 to 1.37) additional days of hospitalization, and incurring $14,852 (95% CI: 12,204 to 17,501) excess hospital charges, p < 0.001.
Conclusion:CFLD is associated with multiple comorbidities and is independently associated with increased risk of mortality and increased health care resource utilization in pediatric CF-related hospitalizations.
“…1 ), some directly related to liver dysfunction and some a consequence of liver dysfunction. People with severe CFLD are known to have endocrine comorbidities compared with matched people with CF without liver disease [33] . Fig.…”
Section: Cfld and General Endocrine Considerationsmentioning
confidence: 99%
“…Bone disease/osteoporosis: Osteoporosis is the predominant bone disorder seen in patients with CFLD. Those with CFLD have a 1.8 fold higher frequency of CF bone disease (CFBD) compared to matched controls with CF [33] . The increased risk of CFBD in those with liver disease is multifactorial and is associated with low BMI, IGF-1 deficiency, hypogonadism, vitamin D and vitamin K deficiencies.…”
Section: Cfld and General Endocrine Considerationsmentioning
Highlights
Development of CFLD adds significant morbidity and mortality to those with CF.
CFLD is associated with an increased prevalence of endocrinopathies.
Current treatment options for CFLD remain limited.
Early recognition and treatment of CFLD associated conditions is critical.
“…Patients with CFLD, especially those with a severe phenotype, are at risk of significant malnutrition, micronutrient deficiencies and worse lung function [ 108 ]. The underlying causes are complex, including but not limited to poor intake associated with anorexia, delayed gastric emptying, and early satiety from abdominal distension (ascites, organomegaly); increased energy expenditure; and maldigestion/malabsorption [ 109 ].…”
Cystic fibrosis (CF) is a chronic, multisystem disease with multiple comorbidities that can significantly affect nutrition and quality of life. Maintaining nutritional adequacy can be challenging in people with cystic fibrosis and has been directly associated with suboptimal clinical outcomes. Comorbidities of CF can result in significantly decreased nutritional intake and intestinal absorption, as well as increased metabolic demands. It is crucial to utilize a multidisciplinary team with expertise in CF to optimize growth and nutrition, where patients with CF and their loved ones are placed in the center of the care model. Additionally, with the advent of highly effective modulators (HEMs), CF providers have begun to identify previously unrecognized nutritional issues, such as obesity. Here, we will review and summarize commonly encountered comorbidities and their nutritional impact on this unique population.
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